The Jamaican Haemophilia Registry: Describing the burden of disease. Issue 4 (31st May 2018)

• Record Type: Journal Article
• Authors: Wharfe, G.; Buchner‐Daley, L.; Gibson, T.; Hilliard, P.; Usuba, K.; Abad, A.; Boma‐Fischer, L.; Bouskill, V.; Floros, G.; Lillicrap, D.; Lowe, Y.; Lowe, D.; Palmer‐Mitchell, N.; Rand, M. L.; Teitel, J.; Tuttle, A.; Watson, A.; White, R.; Young, N. L.; Blanchette, V. S.
• Is Part Of: Haemophilia. Volume 24:Issue 4(2018)
• Page Start: e179
• Page End: e186
• Publication Date: 2018-05-31
• Subjects: burden of disease -- haemophilia -- health assessment -- Jamaica -- outcome measures -- registry; Hemophilia -- Periodicals; 616.1572005
• DOI: 10.1111/hae.13517
• Languages: English
• ISSNs: 1351-8216

Abstract

Abstract : Introduction: Jamaica has an estimated 200 persons with haemophilia (PWH), who face significant constraints in access to specialized haemophilia care, including access to clotting factor concentrates. Aim: The aim of this paper is to establish the current burden of disease in PWH in Jamaica. Methods: PWH were enrolled through the University Hospital of the West Indies, Jamaica. The impact of haemophilia was assessed using a comprehensive battery of heath outcome measures that included the following: laboratory, clinical information and validated outcome measures of joint structure and function, activity, and health‐related quality of life (HRQoL) to provide a health profile of the Jamaican haemophilia population. Results: In all, 45 PWH were registered (mean age: 29, range: 0.17‐69 years), including 13 children (<18 years of age) and 32 adults. In this sample, 41 had haemophilia A (30 severe) and 4 had haemophilia B (3 severe); 10 patients with haemophilia A were inhibitor positive. The results indicate that adults with haemophilia in Jamaica have significant joint damage: mean Haemophilia Joint Health Score (HJHS) = 42.1 (SD = 17.3); moderate activity levels − mean Haemophilia Activities List (HAL) score = 64.8 (SD = 17.8); and low HRQoL scores − mean Haemo‐QoL‐A score = 62.3 (SD = 19.4). Results for children are also reported but should be interpreted with caution due to the small sample size. Conclusions: There is a very high burden of disease in PWH in Jamaica. The health profiles reported in this paper are an essential first step in advocating for a multidisciplinary Comprehensive Care Program for assessment and care of PWH in Jamaica.